Survival From Birth Until Young Adulthood Among Individuals With Congenital Heart Defects: CH STRONG.

BACKGROUND: Limited population-based information is available on long-term survival of US individuals with congenital heart defects (CHDs). Therefore, we assessed patterns in survival from birth until young adulthood (ie, 35 years of age) and associated factors among a population-based sample of US individuals with CHDs. METHODS: Individuals born between 1980 and 1997 with CHDs identified in 3 US birth defect surveillance systems were linked to death records through 2015 to identify those deceased and the year of their death. Kaplan-Meier survival curves, adjusted risk ratios (aRRs) for infant mortality (ie, death during the first year of life), and Cox proportional hazard ratios for survival after the first year of life (aHRs) were used to estimate the probability of survival and associated factors. Standardized mortality ratios compared infant mortality, >1-year mortality, >10-year mortality, and >20-year mortality among individuals with CHDs with general population estimates. RESULTS: Among 11 695 individuals with CHDs, the probability of survival to 35 years of age was 81.4% overall, 86.5% among those without co-occurring noncardiac anomalies, and 92.8% among those who survived the first year of life. Characteristics associated with both infant mortality and reduced survival after the first year of life, respectively, included severe CHDs (aRR=4.08; aHR=3.18), genetic syndromes (aRR=1.83; aHR=3.06) or other noncardiac anomalies (aRR=1.54; aHR=2.53), low birth weight (aRR=1.70; aHR=1.29), and Hispanic (aRR=1.27; aHR=1.42) or non-Hispanic Black (aRR=1.43; aHR=1.80) maternal race and ethnicity. Individuals with CHDs had higher infant mortality (standardized mortality ratio=10.17), >1-year mortality (standardized mortality ratio=3.29), and >10-year and >20-year mortality (both standardized mortality ratios ≈1.5) than the general population; however, after excluding those with noncardiac anomalies, >1-year mortality for those with nonsevere CHDs and >10-year and >20-year mortality for those with any CHD were similar to the general population. CONCLUSIONS: Eight in 10 individuals with CHDs born between1980 and 1997 survived to 35 years of age, with disparities by CHD severity, noncardiac anomalies, birth weight, and maternal race and ethnicity. Among individuals without noncardiac anomalies, those with nonsevere CHDs experienced similar mortality between 1 and 35 years of age as in the general population, and those with any CHD experienced similar mortality between 10 and 35 years of age as in the general population.

Adverse Effect of Bundle Branch Block on Exercise Performance in Patients with Fontan Physiology: From the Pediatric Heart Network Fontan Public Data Set.

Patients with Fontan physiology have reduced exercise performance compared to their peers as well as a higher incidence of bundle branch block (BBB). This study aims to investigate the association between BBB and exercise performance in the Fontan population through a retrospective review of the Pediatric Heart Network Fontan study public use dataset. "Low Performers" were defined as ≤ 25th percentile (for Fontan patients) for each exercise parameter at anaerobic threshold (AT) for gender and age and "Normal Performers" were all other patients. A total of 303 patients with Fontan physiology who underwent exercise testing reached AT and had complete data for BBB. BBB occurred more frequently in Low Performers for VO2 [OR (95% CI): 2.6 (1.4, 4.8)] and Work [OR (95% CI): 2.7 (1.4, 5.1)], suggesting that BBB in the Fontan population is associated with reduced exercise performance. This data adds to the existing clinical evidence of the adverse effects of conduction abnormalities on single ventricle cardiac output and adds support for consideration of cardiac resynchronization and multi-site ventricular pacing in this patient population.

Congenital left atrial appendage ostial stenosis in an extremely premature infant diagnosed by transthoracic echocardiography.

Congenital left atrial appendage ostial stenosis is a very rare congenital cardiac condition. We present the case of an extremely premature infant with congenital left atrial appendage ostial stenosis diagnosed by transthoracic echocardiographic imaging.

Health Insurance Status and Access to Healthcare Among Young Adults with Congenital Heart Disease: from the Congenital Heart Survey To Recognize Outcomes, Needs and Well-beinG (CH STRONG), 2016-2019.

Having health insurance is associated with better access to healthcare and lower rates of comorbidity in the general population, but data are limited on insurance's impact on adults with congenital heart disease (ACHD). The Congenital Heart Survey To Recognize Outcomes, Needs and well-beinG (CH STRONG) was conducted among ACHD in three locations from 2016 to 2019. We performed multivariable logistic regression to determine the associations between health insurance and both access to healthcare and presence of comorbidities. We also compared health insurance and comorbidities among ACHD to similarly-aged individuals in the Behavioral Risk Factor Surveillance System (BRFSS) as a proxy for the general population. Of 1354 CH STRONG respondents, the majority were ≤ 30 years old (83.5%), and 8.8% were uninsured versus 17.7% in the BRFSS (p < 0.01). Compared to insured ACHD, uninsured were less likely to report regular medical care (adjusted odds ratio [aOR] 0.2, 95% confidence interval [CI] 0.1-0.3) and visited an emergency room more often (aOR 1.6, CI 1.0-2.3). Among all ACHD reporting disability, uninsured individuals less frequently received benefits (aOR 0.1, CI 0.0-0.3). Depression was common among uninsured ACHD (22.5%), but insured ACHD had lower rates of depression than insured in the BRFSS (13.3% vs. 22.5%, p < 0.01). In conclusion, rates of insurance were higher among ACHD compared to the general population. Nonetheless, uninsured ACHD inconsistently accessed healthcare and benefits. Further studies are needed to determine if insurance ameliorates the risk of morbidity as ACHD age.

Worse Hospital Outcomes for Children and Adults with COVID-19 and Congenital Heart Disease.

The aim of the current study is to investigate hospitalization outcomes of COVID-19 positive children and adults with moderate or severe congenital heart disease to children and adults without congenital heart disease. Retrospective review using the Vizient Clinical Data Base for admissions of patients with an ICD-10 code for COVID-19 from April 2020 to March 2021. Admissions with COVID-19 and with and without moderate or severe congenital heart disease (CHD) were stratified into pediatric (< 18 years) and adult (≥ 18 years) and hospital outcomes were compared. There were 9478 pediatric COVID-19 admissions, 160 (1.7%) with CHD, and 658,230 adult COVID-19 admissions, 389 (0.06%) with CHD. Pediatric admissions with COVID-19 and CHD were younger (1 vs 11 years), had longer length of stay (22 vs 6 days), higher complication rates (6.9 vs 1.1%), higher mortality rates (3.8, 0.8%), and higher costs ($54,619 vs 10,731; p < 0.001 for all). Adult admissions with COVID-19 and CHD were younger (53 vs 64 years, p < 0.001), had longer length of stay (12 vs 9 days, p < 0.001), higher complication rates (8 vs 4.8%, p = 0.003), and higher costs ($23,551 vs 13,311, p < 0.001). This appears to be the first study to report the increased hospital morbidities and costs for patients with CHD affected by COVID-19. Our hope is that these findings will help counsel patients moving forward during the pandemic.

Advance Care Directives Among a Population-Based Sample of Young Adults with Congenital Heart Defects, CH STRONG, 2016-2019.

Little is known about advance care planning among young adults with congenital heart defects (CHD). Congenital Heart Survey to Recognize Outcomes, Needs, and well-beinG (CH STRONG) participants were born with CHD between 1980 and 1997, identified using active, population-based birth defects surveillance systems in Arkansas, Arizona and Atlanta, and Georgia, and surveyed during 2016-2019. We estimated the percent having an advance care directive standardized to the site, year of birth, sex, maternal race, and CHD severity of the 9312 CH STRONG-eligible individuals. We calculated adjusted odds ratios (aOR) and 95% confidence intervals (CI) for characteristics associated with having advance care directives. Of 1541 respondents, 34.1% had severe CHD, 54.1% were female, and 69.6% were non-Hispanic white. After standardization, 7.3% had an advance care directive (range: 2.5% among non-Hispanic blacks to 17.4% among individuals with "poor" perceived health). Individuals with severe CHD (10.5%, aOR = 1.6, 95% CI: 1.1-2.3), with public insurance (13.1%, aOR = 1.7, 95% CI: 1.1-2.7), with non-cardiac congenital anomalies (11.1%, aOR = 1.9, 95% CI: 1.3-2.7), and who were hospitalized in the past year (13.3%, aOR = 1.8, 95% CI: 1.1-2.8) were more likely than their counterparts to have advance care directives. Individuals aged 19-24 years (6.6%, aOR = 0.4, 95% CI: 0.3-0.7) and 25-30 years (7.6%, aOR = 0.5, 95% CI: 0.3-0.8), compared to 31-38 years (14.3%), and non-Hispanic blacks (2.5%), compared to non-Hispanic whites (9.5%, aOR = 0.2, 95% CI: 0.1-0.6), were less likely to have advance care directives. Few young adults with CHD had advance care directives. Disparities in advance care planning may exist.

Rationale and design of CH STRONG: Congenital Heart Survey To Recognize Outcomes, Needs, and well-beinG.

Studies of outcomes among adults with congenital heart defects (CHDs) have focused on those receiving cardiac care, limiting generalizability. The Congenital Heart Survey To Recognize Outcomes, Needs, and well-beinG (CH STRONG) will assess comorbidities, health care utilization, quality of life, and social and educational outcomes from a US population-based sample of young adults living with CHD. METHODS: Individuals with CHD born between 1980 and 1997 were identified using active, population-based birth defects surveillance systems from 3 US locations (Arkansas [AR]; Arizona [AZ]; and Atlanta, Georgia [GA]) linked to death records. Individuals with current contact information responded to mailed survey materials during 2016 to 2019. Respondents and nonrespondents were compared using χ2 tests. RESULTS: Sites obtained contact information for 74.6% of the 9,312 eligible individuals alive at recruitment. Of those, 1,656 returned surveys, either online (18.1%) or via paper (81.9%), for a response rate of 23.9% (AR: 18.3%; AZ: 30.7%; Atlanta, GA: 28.0%; P value < .01). For 20.0% of respondents, a proxy completed the survey, with 63.9% reporting that the individual with CHD was mentally unable. Among respondents and nonrespondents, respectively, sex (female: 54.0% and 47.3%), maternal race/ethnicity (non-Hispanic white: 74.3% and 63.0%), CHD severity (severe: 33.8% and 27.9%), and noncardiac congenital anomalies (34.8% and 38.9%) differed significantly (P value < .01); birth year (1991-1997: 56.0% and 57.5%) and presence of Down syndrome (9.2% and 8.9%) did not differ. CONCLUSIONS: CH STRONG will provide the first multisite, population-based findings on long-term outcomes among the growing population of US adults with CHD.

Early bioprosthetic tricuspid valve stenosis due to size mismatch in Ebstein anomaly-Successful transcatheter treatment.

Patients with congenital heart disease are surviving well into adulthood thanks to advances in medical and clinical care. We present a patient with Ebstein anomaly who underwent surgical tricuspid valve replacement and suffered early valve stenosis due to her unique anatomy. This case highlights the importance of the "unnatural" anatomy that can be encounter in this challenging patient population.

Effect of comorbid neuropsychiatric disorders on children and adolescents undergoing surgery for moderate and severe congenital heart disease.

BACKGROUND: Children and adolescents with congenital heart disease (CHD) are at an increased risk of neuropsychiatric disorders (NPDs). The purpose of this study is to determine how a comorbid NPD affects hospital outcomes and costs for CHD patients undergoing cardiac surgery. METHODS: Retrospective review of the 2000-2012 Healthcare Cost and Utilization Project Kids' Inpatient Databases for admissions 10 to 21 years old with an ICD-9 code for moderate or severe CHD and a procedure code for cardiopulmonary bypass as a marker for cardiac surgery; admissions with syndromes that could be associated with NPD were excluded. Demographics, hospital outcomes, and charges were compared between admissions with and without NPD ICD-9 codes using analysis of variance, independent samples Kruskal-Wallis, and χ2 , as appropriate. RESULTS: There were 4768 admissions with CHD and cardiac surgery: 4285 (90%) with no NPD, 93 (2%) with cognitive deficits, 390 (8%) with mood/behavior deficits. Patients with NPD had a longer length of stay and higher mean charges (P < .001 for both). Patients with mood/behavior deficits were older and patients with cognitive deficits were more likely female (P < .001 for both). CONCLUSIONS: Children and adolescents with moderate or severe CHD and NPD who undergo cardiac surgery incur longer hospital stays and higher charges. Recognizing and addressing the underlying NPDs may be important to improve postoperative progression for children and adolescents with CHD hospitalized for cardiac surgery.

Treatment of tricuspid regurgitation and para-ring leak in tetralogy of Fallot with oversized SAPIEN 3 valve-in-ring implantation.

A 40-year-old female with previously repaired tetralogy of Fallot had recurrent severe tricuspid regurgitation with a para-ring leak after annuloplasty ring placement. Because of multiple prior sternotomies and co-morbidities, she was not felt to be a surgical candidate. Percutaneous placement of an oversized SAPIEN 3 valve-in-ring in the tricuspid position successfully treated the regurgitation and para-ring leak.

Higher Cost of Hospitalizations for Non-cardiac Diagnoses in Adults with Congenital Heart Disease.

Adults with congenital heart disease (CHD) are a rapidly increasing population and their impact on healthcare resources is not fully understood. The purpose of this study was to describe the costs of hospitalizations for non-cardiac disease for adults with CHD. We conducted a retrospective review of hospital discharge data from the University HealthSystem Consortium Clinical Data Base/Resource Manager from January 2011 through December 2013. Patients were ≥ 18 years old at admission with any ICD-9 code for moderate or high severity CHD; cardiac surgical admissions were excluded. The comparison group consisted of patients ≥ 18 years old with no ICD-9 codes for any severity CHD. There were 9,169,700 non-CHD, 28,224 moderate CHD, and 3045 high severity CHD hospital admissions. Total length of stay was longer for acute kidney injury, depressive disorder, esophageal reflux, and obstructive sleep apnea for any severity CHD; ICU admission rates were higher for all diagnoses with any severity CHD. Mean observed direct costs were higher for all diagnoses for moderate CHD and all diagnoses except dehydration, type 2 diabetes, obesity, and obstructive sleep apnea for high severity CHD. This review identified significantly increased hospitalization costs for adults with moderate and high severity CHD who are admitted for non-cardiac medical conditions not associated with concomitant cardiac surgical procedures. Admissions with CHD diagnoses had higher ICU admission rates, longer lengths of stay, and higher mortality for most non-cardiac admission diagnoses. These data will add to our understanding of the economic impact of adults with CHD.

Decreased false-positive adolescent pre-athletic screening with Seattle Criteria-interpreted electrocardiograms.

Sudden cardiac arrest is a rare but devastating cause of death in young adults. Electrocardiograms may detect many causes of sudden cardiac arrest, but are not routinely included in pre-athletic screening in the United States of America partly because of high rates of false-positive interpretation. To improve electrocardiogram specificity for identifying cardiac conditions associated with sudden cardiac arrest, an expert panel developed refined criteria known as the Seattle Criteria. Ours is the first study to compare standard electrocardiogram criteria with Seattle Criteria in 11- to 13-year-olds. In total, 1424 students completed the pre-athletic screening and electrocardiogram; those with a positive screen or abnormal electrocardiogram interpreted by a paediatric electrophysiologist completed further work-up. Electrocardiograms referred for additional evaluation were re-interpreted by a paediatric electrophysiologist using Seattle Criteria. Electrocardiogram abnormalities were identified in 98 (6.9%); Seattle Criteria identified 28 (2.0%). Formal evaluation confirmed four students at risk for sudden cardiac arrest (0.3%): long QT syndrome (n=2), Wolff-Parkinson-White (n=1), and pulmonary hypertension (n=1). All students with at-risk phenotypes for sudden cardiac arrest were identified by both standard electrophysiologist and Seattle Criteria. The false-positive interpretation rate decreased from 6.6 to 1.7% with Seattle Criteria. Downstream costs associated with screening using standard paediatric electrocardiogram interpretations and Seattle Criteria were projected at $24 versus $7, respectively. In conclusion, using Seattle Criteria for electrocardiogram interpretation decreases the rate of false-positive results compared with standard interpretation without omitting true-positive electrocardiogram findings. This may decrease unnecessary referrals and costs associated with formal cardiology evaluation.

Feasibility of pulse oximetry screening for critical congenital heart disease at 2643-foot elevation.

To evaluate the feasibility of implementing a pulse oximetry screening protocol at a city of mild elevation with a specific focus on the false-positive screening rate. Pulse oximetry screening was performed according to the proposed guidelines endorsed by the American Academy of Pediatrics at a center in Tucson, AZ, at an elevation of 2,643 ft (806 m). During a 10-month period in 2012, 1069 full-term asymptomatic newborns were screened ≥ 24 h after birth. The mean preductal oxygen saturation was 98.5 ± 1.3 % (range 92-100 %), and the mean postductal oxygen saturation was 98.6 ± 1.3 % (range 94-100 %). Of 1,069 patients screened, 7 were excluded secondary to protocol violations, and 1 screened positive. An echocardiogram was performed on the newborn with the positive screen, and it was normal with the exception of right-to-left shunting across a patent foramen ovale. The false-positive rate was 1/1,062 or 0.094 %. The pulse oximetry screening guidelines recommended by the American Academy of Pediatrics are feasible at an elevation of 2,643 ft (806 m) with a low false-positive rate. Adjustments to the protocol are not required for centers at elevations ≤ 2,643 ft. Future studies at greater elevations are warranted.

Contemporary pregnancy outcomes for women with moderate and severe congenital heart disease.

Background: Women with congenital heart disease (CHD) are surviving into adulthood, with more undergoing pregnancy. Methods: Retrospective review of the Vizient database from 2017-2019 for women 15-44 years old with moderate, severe or no CHD and vaginal delivery or caesarean section. Demographics, hospital outcomes and costs were compared. Results: There were 2,469,117 admissions: 2,467,589 with no CHD, 1277 with moderate and 251 with severe CHD. Both CHD groups were younger than no CHD, there were fewer white race/ethnicity in the no CHD group and more women with Medicare in both CHD groups compared to no CHD. With increasing CHD severity there was an increase in length of stay, ICU admission rates and costs. There were also higher rates of complications, mortality and caesarean section in the CHD groups. Conclusion: Pregnant women with CHD have more problematic pregnancies and understanding this impact is important to improve management and decrease healthcare utilization.

Reproductive Health Counseling and Concerns Among Women with Congenital Heart Defects With and Without Disabilities.

Background: Women with disabilities are less likely to receive reproductive health counseling than women without disabilities. Yet, little is known about reproductive health counseling and concerns among women with congenital heart defects (CHD) and disabilities. Methods: We used population-based survey data from 778 women aged 19 to 38 years with CHD to examine contraceptive and pregnancy counseling and pregnancy concerns and experiences by disability status, based on six validated questions on vision, hearing, mobility, cognition, self-care, and living independently. Multivariable Poisson regression was used to examine adjusted prevalence ratios between disability status and each outcome, adjusted for CHD severity, age, race/ethnicity, place of birth (Arkansas, Arizona, Georgia), and insurance type. Results: Women with disabilities (n = 323) were 1.4 and 2.3 times more likely than women without disabilities (n = 455) to receive clinician counseling on safe contraceptive methods and avoiding pregnancy because of their CHD. Women with CHD and disabilities, compared to those without disabilities, were more likely to be concerned about their ability to have children (aPR = 1.2) and to have delayed or avoided pregnancy (aPR = 2.2); they were less likely to have ever been pregnant (aPR = 0.7). Associations differed slightly across specific disability types. All associations remained after excluding 71 women with chromosomal anomalies. Conclusion: Among women with CHD, reproductive counseling, concerns, and experiences differ by disability status.

Cardiology Care and Loss to Follow-Up Among Adults With Congenital Heart Defects in CH STRONG.

Many of the estimated 1.4 million adults with congenital heart defects (CHDs) in the United States are lost to follow-up (LTF) despite recommendations for ongoing cardiology care. Using 2016 to 2019 CH STRONG (Congenital Heart Survey To Recognize Outcomes, Needs, and well-beinG) data, we describe cardiac care among community-based adults with CHD, born in 1980 to 1997, identified through state birth defects registries. Our estimates of LTF were standardized to the CH STRONG eligible population and likely more generalizable to adults with CHD than clinic-based data. Half of our sample were LTF and more than 45% had not received cardiology care in over 5 years. Of those who received care, only 1 in 3 saw an adult CHD physician at their last encounter. Not knowing they needed to see a cardiologist, being told they no longer needed cardiology care, and feeling "well" were the top reasons for LTF, and only half of respondents report doctors discussing the need for cardiac follow-up.

Dysregulation of the PDGFRA gene causes inflow tract anomalies including TAPVR: integrating evidence from human genetics and model organisms.

Total anomalous pulmonary venous return (TAPVR) is a congenital heart defect inherited via complex genetic and/or environmental factors. We report detailed mapping in extended TAPVR kindreds and mutation analysis in TAPVR patients that implicate the PDGFRA gene in the development of TAPVR. Gene expression studies in mouse and chick embryos for both the Pdgfra receptor and its ligand Pdgf-a show temporal and spatial patterns consistent with a role in pulmonary vein (PV) development. We used an in ovo function blocking assay in chick and a conditional knockout approach in mouse to knock down Pdgfra expression in the developing venous pole during the period of PV formation. We observed that loss of PDGFRA function in both organisms causes TAPVR with low penetrance (approximately 7%) reminiscent of that observed in our human TAPVR kindreds. Intermediate inflow tract anomalies occurred in a higher percentage of embryos (approximately 30%), suggesting that TAPVR occurs at one end of a spectrum of defects. We show that the anomalous pulmonary venous connection seen in chick and mouse is highly similar to TAPVR discovered in an abnormal early stage embryo from the Kyoto human embryo collection. Whereas the embryology of the normal venous pole and PV is becoming understood, little is known about the embryogenesis or molecular pathogenesis of TAPVR. These models of TAPVR provide important insight into the pathogenesis of PV defects. Taken together, these data from human genetics and animal models support a role for PDGF-signaling in normal PV development, and in the pathogenesis of TAPVR.

Remote diagnosis of congenital heart disease in southern Arizona: comparison between tele-echocardiography and videotapes.

OBJECTIVE: We report our experience with tele-echocardiography and echocardiograms recorded digitally or on videotape (recorded-echos) at The University of Arizona from August 2006 to December 2010 and compare their quality and diagnostic accuracy. MATERIALS AND METHODS: Tele-echocardiograms (tele-echos) were transmitted from the Yuma Regional Medical Center to The University of Arizona via a T-1 and aT-3 line at a bandwidth of 768 kilobits per second. Recorded-echos were shipped for interpretation to The University of Arizona by overnight mail. Diagnostic accuracy was assessed by comparing tele- and recorded-echos with electrocardiograms performed by a pediatric cardiologist (PedsCard-echos). RESULTS: Three hundred forty-six tele-echos in 260 patients and 455 recorded-echos in 406 patients were performed (median age, 6 and 8 days, respectively). Indications included possible congenital heart disease (CHD), patent ductus arteriosus (PDA), and persistent pulmonary hypertension of the newborn. Diagnostic categories included complex CHD, non-critical disease, PDA, and other. PedsCard-echos were available for 27% of the tele-echo and 30% of the recorded-echo patients. Comparisons between tele- and PedsCard-echo yielded no discrepancies in 12 (23%), expected resolution of condition in 26 (49%), and minor in 14 (26%). One (2%) major discrepancy was detected. Comparisons between recorded- and PedsCard-echo showed no discrepancies in 28 (40%), expected resolution of condition in 14 (20%), and minor discrepancies in 28 (40%) patients. No significant difference with respect to discrepancies was detected between tele- and recorded-echos. There was significant (p<0.01) improvement in tele- and recorded-echo study quality by 2010. CONCLUSIONS: (1) Tele-echocardiography can be performed successfully with excellent accuracy. (2) The quality of tele- and recorded-echo studies improved toward the end of the analysis period. (3) Although initially tele-echo studies were more accurate than recorded-echo studies, there was no difference between these two types of studies by the fourth year of the study. (4) Both tele- and recorded-echos were indispensible in the remote diagnosis of CHD.

Heart-valve mesenchyme formation is dependent on hyaluronan-augmented activation of ErbB2-ErbB3 receptors.

Heart septation and valve malformations constitute the most common anatomical birth defects. These structures arise from the endocardial cushions within the atrioventricular canal (AVC) through dynamic interactions between cushion cells and the extracellular matrix (termed cardiac jelly). Transformation of endothelial cells to mesenchymal cells is essential for the proper development of the AVC and subsequent septation and valve formation. Atrioventricular septal defects can result from incomplete endocardial cushion morphogenesis. We show that hyaluronan-deficient AVC explants from Has2(-/-) embryos, which normally lack mesenchyme formation, are rescued by heregulin treatment, which restores phosphorylation of ErbB2 and ErbB3. These events were blocked using a soluble ErbB3 molecule, as well as with an inhibitor of ErbB2, herstatin. We show further that ErbB3 is activated during hyaluronan treatment of Has2(-/-) explants. These data provide a link between extracellular matrix-hyaluronan and ErbB receptor activation during development of early heart-valve and septal mesenchyme.

Disability Among Young Adults With Congenital Heart Defects: Congenital Heart Survey to Recognize Outcomes, Needs, and Well-Being 2016-2019.

Background Disabilities have implications for health, well-being, and health care, yet limited information is available on the percentage of adults with congenital heart defects (CHD) living with disabilities. We evaluated the prevalence of disability and associated characteristics among the 2016-2019 CH STRONG (Congenital Heart Survey to Recognize Outcomes, Needs, and Well-Being) population-based sample of 19- to 38-year-olds with CHD from 3 US locations. Methods and Results Prevalence of disability types (hearing, vision, cognition, mobility, self-care, living independently) were compared with similarly aged adults from the general population as estimated by the American Community Survey and standardized to the CH STRONG eligible population to reduce nonresponse bias and confounding. Health-related quality of life (HRQOL) was measured via Patient-Reported Outcomes Measurement Information System Global Health Scale T-scores standardized to US 18- to 34-year-olds. Separate multivariable regression models assessed associations between disability and HRQOL. Of 1478 participants, 40% reported disabilities, with cognition most prevalent (29%). Of those reporting disability, 45% ever received disability benefits and 46% were unemployed. Prevalence of disability types were 5 to 8 times higher in adults with CHD than the general population. Those with ≥1 disability had greater odds of being female, and of having non-Hispanic Black maternal race and ethnicity, severe CHD, recent cardiac care, and noncardiac congenital anomalies. On average, adults with CHD and cognition, mobility, and self-care disabilities had impaired mental HRQOL and those with any disability type had impaired physical HRQOL. Conclusions Two of 5 adults with CHD may have disabilities, which are associated with impaired HRQOL. These results may inform healthcare needs and services for this growing population.